Pulmonary hypertension is a disorder in which the blood vessels in the lungs become more and more narrow. As a result, less blood reaches the air sacs (alveoli) in the lungs, hampering oxygen uptake. People with PH develop a markedly decreased exercise capacity and have a reduced quality of life. The most common symptoms of PH include shortness of breath, fatigue, dizziness and fainting, all of which are worsened by exertion.
When the air gets thinner
Pulmonary hypertension (PH) is a severe, progressive, life-changing and life-threatening disorder of the lungs and heart in which the blood pressure in the pulmonary arteries is above normal and which can lead to right heart failure and death. Patients develop high blood pressure in the arteries of the lungs, which causes breathlessness, fatigue and hinders their ability to work and carry out everyday activities, such as walking short distances or climbing stairs. PH can affect people of all ages, including children, though the average age at diagnosis is approximately 50 years. It is thought that there are several hundred thousand patients globally diagnosed with PH. There are five types of PH, and each can affect the patient in a different way.
Fatal constriction of the blood vessels
The feeling for those affected is like being at an altitude of several thousand meters. It is as though the air were getting thinner – all the time. As a result, the heart is permanently fighting an uphill battle against the resistance caused by constricted blood vessels – until it finally fails.
Up until a few years ago, a lung transplant was the only hope for patients with pulmonary hypertension. For several years now, Bayer has been providing a drug that can halt the progression of one form of pulmonary hypertension, namely pulmonary arterial hypertension (PAH). It is inhaled as a fine mist directly into the lungs. Once there, the active substance expands the blood vessels in the lungs, thus improving the circulation of blood and oxygen uptake. This increases the patients' functional capacity and quality of life and extends their survival time. The drug's active substance is often used as part of a combination therapy.
The correct diagnosis often comes late
As the symptoms of PH are non-specific, diagnosis can be delayed by as much as two years. For the best chance of success patients need to be treated at a PH specialist center. An echocardiogram of the right side of the heart, which indirectly measures the pressure in the lungs, can provide important clues. The findings can then be confirmed by a catheter examination of the right heart.
Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension (PAH) is characterized by morphological changes in the endothelium of the arteries of the lungs causing remodeling of the tissue and vasoconstriction, which results in the blood pressure in the pulmonary arteries to be significantly increased. As a result of these changes, the blood vessels in the lungs constrict, making it more difficult for the heart to pump blood through to the lungs. Until recently, pharmacological treatments were only available to treat this form of pulmonary hypertension.
Chronic Thromboembolic Pulmonary Hypertension
Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive and life-threatening disease in which thromboembolic occlusion (fibrotic organized blood clots) of pulmonary vessels gradually leads to increased blood pressure in the pulmonary arteries, resulting in an overload of the right heart. CTEPH may evolve after prior episodes of pulmonary embolism (PE) but the pathogenesis is not completely understood. CTEPH is the only type of PH which has a potential cure, which is surgery. However, CTEPH is inoperable in up to 40 percent of patients and up to 35 percent of patients have persistent or recurrent CTEPH following surgery. These patients need approved pharmacological therapies to manage their disease.
New Drugs Can Improve the Quality of Life
At Bayer, scientists discovered direct stimulators of sGC, a new way to widen the blood vessels, to lower blood pressure in the lungs and to relieve the heart. The first member of this distinct class of medicines is the first drug therapy to be approved in two forms of pulmonary hypertension. Its distinct mode of action has the potential to overcome a number of limitations of previously approved treatments for pulmonary arterial hypertension (PAH) and it is the first drug therapy to become available for another type of PH, inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent or recurrent CTEPH after surgical treatment.
The pivotal Phase III studies showed that the drug was well-tolerated and effective in significantly improving the severity of disease in patients with PAH and inoperable or persistent or recurrent CTEPH after surgery, and reducing many of their symptoms as well as improving quality of life.
Advice for patients
Each body reacts differently to medicines. Therefore it is impossible to tell which medicine works best for you. Please consult your physician.